Imaging of pediatric cardiac tumors: A COG Diagnostic Imaging Committee/SPR Oncology Committee White Paper.

Cardiac tumors in children are rare and the majority are benign. The most common cardiac tumor in children is rhabdomyoma, usually associated with tuberous sclerosis complex. Other benign cardiac masses include fibromas, myxomas, hemangiomas, and teratomas. Primary malignant cardiac tumors are exceedingly rare, with the most common pathology being soft tissue sarcomas. This paper provides consensus-based imaging recommendations for the evaluation of patients with cardiac tumors at diagnosis and follow-up, including during and after therapy.

The ductus arteriosus: a review of embryology to intervention.

This article reviews the physiology of the ductus arteriosus, the pathophysiology of the patent ductus arteriosus (PDA), and the role advanced imaging such as computed tomography (CT) and magnetic resonance imaging (MRI) can play in guiding diagnosis and percutaneous or surgical intervention. A PDA can have variable clinical and radiologic presentations and can be important to characterize in patients with vascular rings, aortic maldevelopment and congenital heart disease. An understanding of the PDA and the application of CT and MRI can allow the radiologist to provide key information to physicians who plan to close a PDA or maintain PDA patency in the setting of ductal-dependent congenital heart disease.

Prospective study of quantitative liver MRI in cystic fibrosis: feasibility and comparison to PUSH cohort ultrasound.

BACKGROUND: Pediatric radiologists can identify a liver ultrasound (US) pattern predictive of progression to advanced liver disease. However, reliably discriminating these US patterns remains difficult. Quantitative magnetic resonance imaging (MRI) may provide an objective measure of liver disease in cystic fibrosis (CF). OBJECTIVE: The purpose of this study was to determine if quantitative MRI, including MR elastography, is feasible in children with CF and to determine how quantitative MRI-derived metrics compared to a research US. MATERIALS AND METHODS: A prospective, multi-institutional trial was performed evaluating CF participants who underwent a standardized MRI. At central review, liver stiffness, fat fraction, liver volume, and spleen volume were obtained. Participants whose MRI was performed within 1 year of US were classified by US pattern as normal, homogeneous hyperechoic, heterogeneous, or nodular. Each MRI measure was compared among US grade groups using the Kruskal-Wallis test. RESULTS: Ninety-three participants (51 females [54.8%]; mean 15.6 years [range 8.1-21.7 years]) underwent MRI. MR elastography was feasible in 87 participants (93.5%). Fifty-eight participants had an US within 1 year of MRI. In these participants, a nodular liver had significantly higher stiffness (P<0.01) than normal or homogeneous hyperechoic livers. Participants with a homogeneous hyperechoic liver had a higher fat fraction (P<0.005) than others. CONCLUSION: MR elastography is feasible in children with CF. Participants with a nodular pattern had higher liver stiffness supporting the US determination of advanced liver disease. Participants with a homogeneous hyperechoic pattern had higher fat fractions supporting the diagnosis of steatosis.

Cardiac Computed Tomographic Angiography Evaluation of Right Ventricle-Pulmonary Artery Conduits with Surgical Correlation.

Right ventricle-pulmonary artery (RV-PA) conduits are used in the treatment of certain congenital heart disease (CHD). RV-PA conduit complications might develop over time and require intervention. To evaluate how well cardiac computed tomographic angiography (CCTA) performs compared to transthoracic echocardiography (TTE) in evaluating RV-PA conduit complications by using surgical findings as the reference standard. A retrospective chart review of all patients over a 5-year period who underwent CCTA for RV-PA conduit evaluation was performed. Patient demographics and clinical data were recorded. Preoperative CCTA and TTE findings were compared to the operative findings for concordance or discordance. Forty-one patients were included, 51% females. The complications were conduit stenosis (28.68%), infection (7.17%) and aneurysm/pseudoaneurysm (6.15%). TTE and CCTA were consistently able to visualize focal conduit stenosis (96%). The greatest discrepancy between TTE and CCTA was in evaluating for aneurysm/pseudoaneurysm, where TTE detected only 2/6 (33%) compared to CCTA which detected 6/6 (100%) of the cases. However, TTE was slightly better at detecting conduit infection (3/7, 43%) compared to CCTA (2/7, 29%). Note that 5 out of 7 patients with endocarditis had bovine jugular graft. CCTA and TTE provide similar diagnostic accuracy evaluating certain types of RV-PA conduit complications. However, certain complications were only visualized on CCTA or TTE making both modalities complementary to each other during diagnostic evaluation.

Intramural course of an anomalous left coronary artery is not always associated with a slit-like ostium.

Anomalous aortic origin of the left coronary artery from an incorrect aortic sinus has been reported as the second most common causes of sudden cardiac arrest in young athletes. Intramural course of the proximal left coronary artery is considered a high-risk morphology. It is associated with a slit-like ostium and elliptical shape of the proximal artery. In this case, all pre-operative cardiac images demonstrated a round ostium and round luminal shape of the left coronary artery, which suggested no evidence of intramural course. On intraoperative inspection, although the ostium and proximal left coronary artery appeared round, the patient had a long intramural course of the left coronary artery which our surgeons performed a successful unroofing procedure. The post-operative images showed a patent new ostium from the correct aortic sinus. These findings are very unusual and proved that we should not exclude intramural course even in the absence of a slit-like ostium and elliptical shape of the proximal left coronary artery.

Evolution of surgical repair of intraseptal anomalous left coronary artery with myocardial ischaemia.

BACKGROUND: Anomalous aortic origin of the left coronary artery with intraseptal course is a rare coronary anomaly associated with an increased risk of myocardial ischaemia. The role and techniques for surgical intervention are evolving, with numerous novel surgical techniques for this challenging anatomy reported in the last 5 years. We report our single-centre experience with surgical repair of intraseptal anomalous left coronary artery in the paediatric population, including clinical presentation, evaluation, and short- to mid-term outcomes. METHODS: All patients with coronary anomalies presenting to our institution undergo standardised clinical evaluation. Five patients aged 4 to 17 years underwent surgical intervention for intraseptal anomalous aortic origin of the left coronary artery between 2012 and 2022. Surgical techniques included coronary artery bypass grafting (n = 1), direct reimplantation with limited supra-arterial myotomy via right ventriculotomy (n = 1), and transconal supra-arterial myotomy with right ventricular outflow tract patch reconstruction (n = 3). RESULTS: All patients had evidence of haemodynamically significant coronary compression, and three had evidence of inducible myocardial ischaemia pre-operatively. There were no deaths or major complications. Median follow-up was 6.1 months (range 3.1-33.4 months). Patients who underwent supra-arterial myotomy (with or without reimplantation) had improved coronary flow and perfusion based on stress imaging and catheterisation data. CONCLUSIONS: Surgical approaches to intraseptal anomalous left coronary artery with evidence of myocardial ischaemia continue to evolve, with new techniques demonstrating promising improvement in coronary perfusion. Further studies are warranted to determine long-term outcomes and refine indications for repair.

How we approach the diagnosis of a vascular anomaly.

Vascular anomalies represent a diverse group of complex disorders that can cause significant complications, including coagulopathies, pain, and decreased function. The diagnosis of vascular anomalies is often challenging due to heterogeneity of presenting phenotypes and overlapping clinical features with other pediatric conditions. Pediatric hematologists/oncologists (PHO) are uniquely positioned for an essential role in diagnosing, managing, and coordinating the multidisciplinary care required to maximize the quality of life of these patients. Here, we review the diagnostic approach involved in patients with vascular anomalies and utilize cases to highlight the challenges involved, and how PHOs can play a vital part in the care of these patients.

Ketamine for treatment of mood disorders and suicidality: A narrative review of recent progress.

BACKGROUND: Mood disorders are a leading cause of morbidity. Many patients experience treatment-resistant depression (TRD), and suicide rates are rising. Faster-acting and more effective antidepressant medications are needed. Four decades of research has transformed the use of ketamine from an anesthetic to an outpatient treatment for major depressive disorder (MDD). Ketamine is a N-methyl-d-aspartate (NMDA) receptor antagonist and has been shown to rapidly improve mood symptoms and suicidal ideation by targeting the glutamate system directly. METHODS: We used the PubMed database to identify relevant articles published until September 1, 2020. We focused on meta-analyses, randomized controlled trials, and original observational studies. We included relevant studies for depression, MDD, TRD, bipolar disorder, anxiety, posttraumatic stress disorder (PTSD), suicide, ketamine, and esketamine. RESULTS: Both racemic ketamine and esketamine have been shown to rapidly treat depression and suicidality. There is evidence that ketamine can be helpful for anxiety and PTSD; however, more research is needed. Intranasal esketamine has been FDA approved to treat depression. CONCLUSIONS: This narrative review describes the evolution of ketamine to treat mood disorders and suicidality. We provide the evidence supporting recent developments using esketamine as well as unresolved issues in the field, such as dosing and safety.

Health-related Quality of Life in a Prospective Study of Ultrasound to Detect Cystic Fibrosis-related Liver Disease in Children.

OBJECTIVES: Cystic fibrosis liver disease (CFLD) begins early in life. Symptoms may be vague, mild, or nonexistent. Progressive liver injury may be associated with decrements in patient health before liver disease is clinically apparent. We examined Health-Related Quality of Life (HRQOL) in children enrolled in a multi-center study of CFLD to determine the impact of early CFLD on general and disease-specific QOL. METHODS: Ultrasound (US) patterns of normal (NL), heterogeneous (HTG), homogeneous (HMG), or nodular (NOD) were assigned in a prospective manner to predict those at risk for advanced CFLD. Parents were informed of results. We assessed parent/child-reported (age ≥5 years) HRQOL by PedsQL 4.0 Generic Core and CF Questionnaire-revised (CFQ-R) prior to US and annually. HRQOL scores were compared by US pattern at baseline (prior to US), between baseline and 1 year and at 5 years. Multivariate analysis of variance (MANOVA) with Hotelling-Lawley trace tested for differences among US groups. RESULTS: Prior to US, among 515 participants and their parents there was no evidence that HTG or NOD US was associated with reduced PedsQL/CFQ-R at baseline. Parents of NOD reported no change in PedsQL/CFQ-R over the next year. Child-report PedsQL/CFQ-R (95 NL, 20 NOD) showed improvement between baseline and year 5 for many scales, including Physical Function. Parents of HMG children reported improved CFQ-R scores related to weight. CONCLUSIONS: Early undiagnosed or pre-symptomatic liver disease had no impact on generic or disease-specific HRQoL, and HRQoL was remarkably stable in children with CF regardless of liver involvement.

Biomarkers for liver disease in urea cycle disorders.

BACKGROUND: Urea cycle disorders (UCDs) are among the most common inborn errors of liver metabolism. As therapies for hyperammonemia associated with urea cycle dysfunction have improved, chronic complications, such as liver disease, have become increasingly apparent in individuals with UCDs. Liver disease in UCDs may be associated with hepatic inflammation, hepatic fibrosis, portal hypertension, liver cancer and even liver failure. However, except for monitoring serum aminotransferases, there are no clear guidelines for screening and/or monitoring individuals with UCDs for liver disease. Thus, we systematically evaluated the potential utility of several non-invasive biomarkers for liver fibrosis in UCDs. METHODS: We evaluated grey-scale ultrasonography, liver stiffness obtained from shear wave elastography (SWE), and various serum biomarkers for hepatic fibrosis and necroinflammation, in a cohort of 28 children and adults with various UCDs. RESULTS: Overall, we demonstrate a high burden of liver disease in our participants with 46% of participants having abnormal grey-scale ultrasound pattern of the liver parenchyma, and 52% of individuals having increased liver stiffness. The analysis of serum biomarkers revealed that 32% of participants had elevated FibroTest™ score, a marker for hepatic fibrosis, and 25% of participants had increased ActiTest™ score, a marker for necroinflammation. Interestingly, liver stiffness did not correlate with ultrasound appearance or FibroTest™. CONCLUSION: Overall, our results demonstrate the high overall burden of liver disease in UCDs and highlights the need for further studies exploring new tools for identifying and monitoring individuals with UCDs who are at risk for this complication. TRIAL REGISTRATION: This study has been registered in ClinicalTrials.gov (NCT03721367).

Transarterial Radioembolization Treatment as a Bridge to Surgical Resection in Pediatric Hepatocellular Carcinoma.

BACKGROUND: Children with unresectable hepatocellular carcinoma (HCC) have a poor prognosis and limited treatment options. Transarterial radioembolization (TARE) using Yttrium-90 (Y90) has emerged as a potential bridge therapy to hepatic resection or transplantation for HCC with very limited studies in children. OBSERVATIONS: Here we present the clinical course of 2 children successfully treated with TARE Y90 for initially unresectable fibrolamellar HCC (FL-HCC) and bridged to partial hemihepatectomy with >1-year overall survival post-TARE. CONCLUSION: Although there have been prior published reports of pediatric patients with HCC being treated with TARE Y90 and some being able to undergo subsequent orthotopic liver transplantation, this is the first report of pediatric HCC patients treated with TARE Y90 as a bridge to nontransplant resections and going on to have >1-year overall survival.

Accuracy of computed tomography angiography and structured reporting of high-risk morphology in anomalous aortic origin of coronary artery: comparison with surgery.

BACKGROUND: Morphological features including interarterial course, intramural course, high ostial location and slit-like ostium are presumed risk factors for sudden cardiac death in children with anomalous aortic origin of the coronary artery (AAOCA). To facilitate clinical risk stratification, the diagnostic accuracy of CT angiography for individual risk factors in the setting of AAOCA must be established. OBJECTIVE: We assessed diagnostic accuracy of standardized CT angiography interpretation for morphological characteristics that might determine risk in children with AAOCA by comparing them to surgical findings. MATERIALS AND METHODS: We created a standardized protocol for CT angiography of AAOCA and retrospectively evaluated diagnostic performance in 25 consecutive surgical patients. Relevant morphological variables in AAOCA were assessed by three independent blinded readers, with surgery as the reference standard. We used Cohen kappa coefficients and accuracies to assess agreement between readers and surgical findings, and we calculated intraclass correlation coefficients to compare length of the intramural course. RESULTS: CT angiography correctly identified AAOCA in all patients. For the three readers, accuracies for detecting ostial stenosis were 84%, 94% and 96%; for high ostial origin, accuracies were 76%, 78% 82%; for intramurality using the peri-coronary fat sign, accuracies were 98%, 96% and 92%; and for intramurality using oval shape of coronary artery, accuracies were 98%, 94% and 92%. The intraclass correlation coefficients (ICCs) for predicting intramural length among the three readers were 0.67, 0.75 and 0.81 using peri-coronary fat, and 0.69, 0.50 and 0.81 using oval shape, respectively. CONCLUSION: CT angiography reliably identified AAOCA in all children and detected the presence of intramurality with high accuracy.

Role of gated cardiac computed tomographic angiography in the evaluation of postsurgical complications after stage I Norwood procedure and its implications on management: a comparative study with two-dimensional echocardiography.

BACKGROUND: The Norwood procedure is the first part of a three-stage surgical palliation for patients with functionally single ventricle anatomy. Complications after the stage I operation are not uncommon. Transthoracic echocardiography (TTE) is traditionally the mainstay for evaluation. OBJECTIVE: The purpose of our study is to compare gated cardiac computed tomographic angiography (CCTA) with TTE when evaluating for postoperative complications after stage I Norwood procedure and to describe management implications. MATERIALS AND METHODS: A retrospective chart review of all patients over a 4-year period who underwent nonelective urgent CCTA for suspected complications related to stage I Norwood procedure was performed. Elective CCTA studies before stage II palliation were excluded. Patient demographics, CCTA and TTE findings, as well as interventions performed, were recorded. RESULTS: Thirty-four patients were included. The mean age at CCTA was 63 days (range: 4-210 days). All patients had a recent TTE with a mean time interval between TTE and CCTA of 2 days. CCTA detected 56 abnormalities in 30 patients, with 23 directly related to postsurgical complications, including shunt-related complications (10/23, 43%), Damus-Kaye-Stansel anastomotic narrowing (2/23, 9%) and neo-aortic arch/branch vessel abnormalities (11/23, 48%). These complications were managed as follows: surgery (9, 39%), catheter-based intervention (7, 30%), medical (4, 17%) and no change in management (3, 13%). TTE did not detect 8/23 (35%) findings found on CCTA, of which 75% were either managed with surgery (4/8, 50%) or catheter-based intervention (2/8, 25%). CONCLUSION: CCTA plays an important role in detecting surgical complications after stage I Norwood procedure and demonstrates additional findings that have direct management implications.

Diagnostic sensitivity and specificity of CT angiography for renal artery stenosis in children.

BACKGROUND: Children with suspected renal artery stenosis (RAS) are screened with renal Doppler ultrasonography or computed tomography (CT) angiography/magnetic resonance (MR) angiography depending on institutional preference. CT angiography produces images with superior resolution, allowing higher quality multiplanar two-dimensional reformats and three-dimensional reconstructions. However, there is a paucity of data in the literature regarding the utility and diagnostic performance of renal CT angiography in pediatric RAS. OBJECTIVE: The objective of this study is to retrospectively review our experience with renal CT angiography in the diagnosis of pediatric RAS relative to digital subtraction angiography (DSA) as the reference standard. MATERIALS AND METHODS: All patients 0-18 years of age who underwent CT angiography for evaluation of RAS as a cause of hypertension between January 2012 and May 2019 were identified for the study. A total of 131 patients were identified, 23 of whom had DSA correlation. RESULTS: Twenty-three patients (17 boys, 6 girls) with a mean age of 6 years 3 months (range: 3 months to 14 years 7 months) were included in this study. Of the 59 renal arteries studied by DSA, 22 were abnormal on CT angiography and 20 were abnormal on DSA. Of the 59 renal arteries, CT angiography was true positive in 18 and true negative in 35. The sensitivity and specificity of CT angiography for RAS diagnosis were 90.0% and 89.7%, respectively. CT angiography identified all cases of main RAS. CONCLUSION: Renal CT angiography has a high sensitivity and specificity for pediatric RAS diagnosis in patients referred for DSA.

Pediatric Hepatoblastoma, Hepatocellular Carcinoma, and Other Hepatic Neoplasms: Consensus Imaging Recommendations from American College of Radiology Pediatric Liver Reporting and Data System (LI-RADS) Working Group.

Appropriate imaging is imperative in evaluating children with a primary hepatic malignancy such as hepatoblastoma or hepatocellular carcinoma. For use in the adult patient population, the American College of Radiology created the Liver Imaging Reporting and Data System (LI-RADS) to provide consistent terminology and to improve imaging interpretation. At present, no similar consensus exists to guide imaging and interpretation of pediatric patients at risk for developing a liver neoplasm or how best to evaluate a pediatric patient with a known liver neoplasm. Therefore, a new Pediatric Working Group within American College of Radiology LI-RADS was created to provide consensus for imaging recommendations and interpretation of pediatric liver neoplasms. The article was drafted based on the most up-to-date existing information as interpreted by imaging experts comprising the Pediatric LI-RADS Working Group. Guidance is provided regarding appropriate imaging modalities and protocols, as well as imaging interpretation and reporting, with the goals to improve imaging quality, to decrease image interpretation errors, to enhance communication with referrers, and to advance patient care. An expanded version of this document that includes broader background information on pediatric hepatocellular carcinoma and rationale for recommendations can be found in Appendix E1 (online).

Heterogeneous Liver on Research Ultrasound Identifies Children with Cystic Fibrosis at High Risk of Advanced Liver Disease: Interim Results of a Prospective Observational Case-Controlled Study.

OBJECTIVE: To assess if a heterogeneous pattern on research liver ultrasound examination can identify children at risk for advanced cystic fibrosis (CF) liver disease. STUDY DESIGN: Planned 4-year interim analysis of a 9-year multicenter, case-controlled cohort study (Prospective Study of Ultrasound to Predict Hepatic Cirrhosis in CF). Children with pancreatic insufficient CF aged 3-12 years without known cirrhosis, Burkholderia species infection, or short bowel syndrome underwent a screening research ultrasound examination. Participants with a heterogeneous liver ultrasound pattern were matched (by age, Pseudomonas infection status, and center) 1:2 with participants with a normal pattern. Clinical status and laboratory data were obtained annually and research ultrasound examinations biannually. The primary end point was the development of a nodular research ultrasound pattern, a surrogate for advanced CF liver disease. RESULTS: There were 722 participants who underwent screening research ultrasound examination, of which 65 were heterogeneous liver ultrasound pattern and 592 normal liver ultrasound pattern. The final cohort included 55 participants with a heterogeneous liver ultrasound pattern and 116 participants with a normal liver ultrasound pattern. All participants with at least 1 follow-up research ultrasound were included. There were no differences in age or sex between groups at entry. Alanine aminotransferase (42 ± 22 U/L vs 32 ± 19 U/L; P = .0033), gamma glutamyl transpeptidase (36 ± 34 U/L vs 15 ± 8 U/L; P < .001), and aspartate aminotransferase to platelet ratio index (0.7 ± 0.5 vs 0.4 ± 0.2; P < .0001) were higher in participants with a heterogeneous liver ultrasound pattern compared with participants with a normal liver ultrasound pattern. Participants with a heterogeneous liver ultrasound pattern had a 9.1-fold increased incidence (95% CI, 2.7-30.8; P = .0004) of nodular pattern vs a normal liver ultrasound pattern (23% in heterogeneous liver ultrasound pattern vs 2.6% in normal liver ultrasound pattern). CONCLUSIONS: Research liver ultrasound examinations can identify children with CF at increased risk for developing advanced CF liver disease.

Pharmacological agents to treat severe neuropsychiatric symptoms in major neurocognitive disorders.

BACKGROUND: Behavioral and psychological symptoms of dementia (BPSD) are challenging to recognize and treat effectively. Despite very effective nonpharmacologic strategies, there are cases where severe BPSD may require biological intervention. The purpose of this review was to clarify safe and evidence-based use of pharmacological agents based on available clinical trial evidence. METHODS: A structured search strategy was used in PubMed, Embase, and PsycInfo to obtain the most relevant data regarding treatment of BPSD in patients with any-cause dementia. RESULTS: We screened 1,442 English-language abstracts. Following initial screening, we had 184 unique references to review. A secondary search of these and review articles gave a total of 117 to tabulate and discuss. We categorized the results based on the following classifications: antidepressants, first-generation antipsychotics, second-generation antipsychotics, anticonvulsants/mood stabilizers, sedatives, stimulants, steroid therapies, other medications, other medication strategies, and medication withdrawal. CONCLUSIONS: The judicious use of pharmacotherapy does seem to be appropriate in select cases where behavioral and psychological interventions have been partially effective or where symptoms confer imminent risk of harm.

Reduced plasma Fetuin-A is a promising biomarker of depression in the elderly.

Depression affects 7% of the elderly population, and it often remains misdiagnosed or untreated. Peripheral biomarkers might aid clinicians by allowing more accurate and well-timed recognition of the disease. We sought to determine if plasma protein levels predict the severity of depressive symptomatology or distinguish patients from healthy individuals. The severity of depressive symptoms and global cognitive functioning were assessed by the Geriatric Depression Scale (GDS) and Mini-Mental State Examination (MMSE) in 152 elderly subjects, 76 of which with major depressive disorder (MDD). Plasma levels of 24 proteins were measured by multiplexing and analyzed as continuous predictors or dichotomized using the median value. The association between individual plasma proteins and MDD risk or depressive symptoms severity was investigated using multiple logistic and linear regressions including relevant covariates. Sensitivity analyses were performed excluding cognitively impaired individuals or non-acute patients with MDD. After adjusting for possible confounders and false discovery rate (FDR) correction, we found lower Fetuin-A levels in MDD patients vs. controls (pFDR = 1.95 × 10-6). This result was confirmed by the sensitivity and dichotomized analyses. Lower prolactin (PRL) levels predicted more severe depressive symptoms in acute MDD patients (pFDR = 0.024). Fetuin-A is a promising biomarker of MDD in the elderly as this protein was negatively associated with the disorder in our sample, regardless of the global cognitive functioning. Lower PRL levels may be a peripheral signature of impaired neuroprotective processes and serotoninergic neurotransmission in more severely depressed patients.

Correlation of ductus arteriosus length and morphology between computed tomographic angiography and catheter angiography and their relation to ductal stent length.

BACKGROUND: Patent ductus arteriosus (PDA) stent placement in infants with ductal-dependent pulmonary blood flow is being increasingly used in clinical practice. OBJECTIVE: To correlate computed tomographic (CT) angiography morphology and length of the PDA with catheter angiography and its relation to eventual PDA stent length. MATERIALS AND METHODS: We retrospectively identified all pediatric patients who underwent PDA stenting at our institute from 2004 to 2018. We included children who had CT angiography prior to stenting. PDA length was measured by a radiologist blinded to the catheter angiography data, using Syngo-via post-processing software (Siemens, Erlangen, Germany). Vessel centerline technique was used. We measured the actual length of the duct as well as straight length between aortic and pulmonary ends. PDA morphology tortuosity index was classified as straight (Type I), mildly tortuous with 1 turn (Type II) and tortuous with >1 turn (Type III), and the PDA origin was noted. The PDA was also measured and morphology classified on catheter angiography by an interventional cardiologist blinded to the CT angiography findings. We compared the CT angiography and catheter angiography lengths, straight lengths and stent length using scatter plots and intraclass correlation coefficient (ICC). RESULTS: A total of 83 children who had PDA stenting were identified, of whom 17 had prior CT angiography. Fifteen of these were neonates. There was agreement between CT angiography and catheter angiography regarding the PDA morphology tortuosity index in 94% of cases and PDA origin in 100% of cases. There was moderate agreement between CT angiography and catheter angiography actual and straight PDA lengths, with ICC coefficients of 0.65 and 0.68, respectively. There was moderate agreement between CT angiography actual length, CT angiography straight length, catheter angiography actual length and eventual stented PDA length, with ICCs of 0.57, 0.67 and 0.73, respectively. There was poor agreement between catheter angiography straight length and eventual stented PDA length, with an ICC of 0.39. CONCLUSION: PDA length and morphology description on CT angiography correlates well with catheter angiography and can be a reliable guide for the interventional cardiologist in decision-making regarding appropriate choice of PDA stent length.

Value of emergent pediatric cardiac computed tomographic angiography service: initial experience at a large children's hospital.

BACKGROUND: Demand for pediatric cardiac computed tomography (CT) angiography is increasing due to recent advances that reduce the need for sedation and radiation exposure while enhancing diagnostic accuracy. This has resulted in the increasing use of cardiac CT angiography emergently during weekends and after hours. The unexpected demand for these services can be challenging, as most hospitals are not staffed to provide 24/7 pediatric cardiovascular imaging. OBJECTIVE: To describe a large single-center experience of providing emergent cardiac CT angiography services in children. MATERIALS AND METHODS: We identified all patients who underwent after--hours weekday and weekend emergent cardiac CT angiography between January 2017 and August 2018. Cardiac CT angiography in the settings of congenital heart disease and coronary imaging were included. Data collected included day and time of cardiac CT angiography, patient age, referral unit, indication, surgical history, need for sedation, need for surgery, intervention and/or change in medical management based on the cardiac CT angiography. RESULTS: Forty-seven studies were identified, 26 (55%) of which were performed on a weekend or holiday and 21 (45%) after 5 p.m. on a weekday. Based on cardiac CT angiography findings, 20 (43%) patients underwent either surgery or an interventional procedure, and 9 (19%) had a change in medical management. The time between cardiac CT angiography and the related surgery/intervention ranged from 0 to 29 days with a median of 3.5 days. CONCLUSION: Emergent pediatric cardiac CT angiography is a valuable service. Larger multi-institutional studies with standardized referral and utilization patterns are needed to determine if outcomes are affected by this service, which in turn will influence hospital staffing patterns for emergent imaging.